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Haematology Flashcards

1
Q

What is the site of haematopoeisis?

A

Bone marrow

Liver

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2
Q

Stat the cells of lymphoid lineage.

A

B cell

T cell

NK Cell

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3
Q

Which cells are derived from the myeloid lineage?

A

Megakaryocyte -> Platelets

Erythrocyte -> RBC

Myeloblast -> Basophil; Neutrophil; Eosinophil; Macrophage

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4
Q

Which blood cells are derived from myeloblasts?

A 
Eosinophils
Basophils
Neutrophils 
Mast cells 
Macrophages
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5
Q

Where do B lymphocytes mature?

A

Bone marrow

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6
Q

Where do T lymphocytes mature?

A

Thymus gland

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7
Q

Anisocytosis is a finding in?

A

Variation in size of RBCs is shown in myelodysplasic syndrome and some forms of anaemia

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8
Q

Target cells are seen in?

A

Central area of pigmentation with pale area and ring of thicker cytoplasm

Seen in iron-deficiency anaemia and post-splenectomy

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9
Q

Howell-Jolly bodies are seen in?

A

Post-splenectomy and severe anaemia (regenerating RBCs quickly)

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10
Q

A high amount of reticulocytes are seen in?

A

Haemolytic anaemia or a condition in which there is a high turnover of RBCs

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11
Q

Schistocytes may appear in?

A

Any condition in which they are being damaged by trauma when travelling through the blood vessels

HUS 
DIC
TTP
Metallic heart valves 
Haemolytic anaemia
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12
Q

In which condition are sideroblasts seen in?

A

Immature RBCs containing iron blobs

Occur when BM unable to incorporate iron into Hb molecules thus indicate a myelodysplastic syndrome

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13
Q

In which condition are smudge cells seen in?

A

Chronic lymphocytic leukaemia

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14
Q

Spherocytes are seen in which condition?

A

Autoimmune haemolytic anaemia

Hereditary spherocytosis

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15
Q

What are the criteria for Anaemia?

State this for M, F and pregnant women.

A

M < 130g/L

F < 120g/L

Pregnant < 110g/L

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16
Q

What are the clinical features of anaemia?

A 
Dyspnoea
Fatigue
Headache
Dizziness
Syncope
Confusion
Palpitations
Angina
Bounding pulse
Postural hypotension
Tachycardia
Conjunctival pallor
Shock
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17
Q

How may anaemia be classified?

A

Morphology:

  • Microcytic
  • Normocytic
  • Macrocytic

Aetiology:

  • Reduced RBC production
  • Increased RBC destruction
  • Blood loss
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18
Q

What are the causes of microcytic anaemia? Give 5 examples.

What is the criteria for a microcytic anaemia?

A 
Iron-deficiency anaemia 
Thalassaemia
Sickle cell disease
Sideroblastic anaemia 
Lead poisoning

<82fL

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19
Q

What are the causes of normocytic anaemia? Give 3 examples.

What is the criteria for a normocytic anaemia?

A

Variable stages of either microcytic or macrocytic anaemia

Anaemia of chronic disease
Blood loss
Renal disease
Pregnancy

82-99fL

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20
Q

What are the causes of macrocytic anaemia? Give 3 examples.

What is the criteria for a macrocytic anaemia?

A 
B12 deficiency
Folate deficiency 
Alcoholism 
Liver disease 
Hypothyroidism
Haematological malignancy 
Reticulocytosis
Drugs - Azathioprine
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21
Q

What are the two categories of Haemolytic Anaemia?

Give 3 examples of each.

A

Inherited vs Acquired

Inherited: 
Hereditary Spherocytosis
Hereditary Elliptocytosis
Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency
Acquired: 
Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related haemolysis
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22
Q

What 3 components of a RBC may be affected in inherited haemolytic anaemia?

A

Tip: 3 components of RBC are membrane, Hb and metabolic machinery. Thus, defect to any of these may cause an inherited haemolytic anaemia.

Membrane: Hereditary spherocytosis

Hb-opathies: Sickle cell anaemia; alpha and ß-thalassaemia

Internal machinery: G6PD deficiency

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23
Q

What is the mechanism of immune-mediated destruction causing haemolytic anaemia?

A

Abs against RBC membrane thus fixing of complement and phagocytosis by macrophages - e.g. AIHA

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24
Q

What is the mechanism of non-immune mediated destruction causing haemolytic anaemia?

A

Various mechanisms related to cause.

Prosthetic heart valve
Microangiopathic haemolytic anaemia 
Infection
Hypersplenism
Drugs
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25
Q

What clinical features would a patient with haemolytic anaemia display on top of the usual anaemia features?

A

Jaundice
Abdominal pain
Dark urine

Neurological signs
Splenomegaly

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26
Q

How is Haptoglobin changed in Haemolytic anaemia?

A

Haptoglobin binds Hb then the Haptoglobin-Hb complex is removed by the liver thus decreasing the Haptoglobin level

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27
Q

A 10 year old patient presents with a normocytic anaemia and some jaundice. It has been going on for a few months now.

What investigations will you conduct?

A 
Commence a haemolytic screen: 
FBC 
LFTs
LDH
Blood film
Haptoglobin

Potentially: DAT; Hb electrophoresis and sickle cell screening

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28
Q

How is Hereditary Spherocytosis inherited?

A

Autosomal dominant

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29
Q

What is the management for a patient with hereditary spherocytosis?

A

Supportive

or

Surgery: Splenectomy

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30
Q

How is Glucose-6-phosphate dehydrogenase deficiency inherited?

A

X-linked

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31
Q

What is the pathophysiology of G6PD deficiency?

A

Glucose 6 phosphate dehydrogenase aids generation of NADPH which is a reducing agent to scavenge oxidative metabolites which can damage RBCs

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32
Q

What is the management of G6PD deficiency?

A

Supportive: Haematology referral; avoid Fava, avoid Henna and numerous drugs

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33
Q

How is Sickle Cell inherited?

A

Autosomal recessive

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34
Q

What are the two types of AIHA? Give examples of each.

A

1) Warm (>37) whereby Abs react against RBCs at higher temperature leading to agglutination.

Idiopathic
Infection 
Chronic inflammation/Inflammatory disorders
Malignancy 
HIV
EBV
Malignancy e.g. CLL; NHL

2) Cold (<32) whereby Abs react against RBCs at lower temperature leading to agglutination.
Idiopathic
M pneumoniae
EBV
Haematological malignancies e.g. Lymphoma

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35
Q

Infection of a patient with hereditary spherocytosis with parvovirus can cause?

A

Aplastic crisis

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36
Q

The presence of Heinz bodies on blood film is indicative of which condition?

A

G6PD deficiency

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37
Q

Give 3 medications which can precipitate G6PD deficiency.

A

Ciprofloxacin
Sulfonylureas
Sulfasalazine
Primaquine (anti-malarial)

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38
Q

Give the two causes of Alloimmune Haemolytic Anaemia.

A

1) In hemolytic transfusion reactions red blood cells are transfused into the patient. The immune system produces antibodies against antigens on those foreign red blood cells. This creates an immune response that leads to the destruction of those red blood cells.
2) In haemolytic disease of the newborn there are antibodies that cross the placenta from the mother to the fetus. These maternal antibodies target antigens on the red blood cells of the fetus. This causes destruction of the red blood cells in the fetus and neonate.

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39
Q

A patient presents with red urine in the morning. They have anaemia and have had several episodes of DVT. Additionally, they experience oesophageal spasms, and have done for years.

What is your diagnosis?

What is the cause of this?

What is the management?

A

Paroxysmal Nocturnal Haemoglobinuria

Mutation in HPSCs in BM result in loss of surface RBCs which inhibit the complement cascade which results in activation of the complement cascade

Eculizumab (C5 mAb)

or

Surgery: BM Transplantation

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40
Q

What is the cause of Pernicious anaemia?

A

Autoimmune condition whereby Antibodies form against parietal cells or IF thus reduced B12 absorption in the terminal ileum

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41
Q

What are the clinical feature of B12 deficiency?

A

Anaemia S+Sx

Neurological symptoms…
Peripheral neuropathy with numbness or paraesthesia (pins and needles)
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive changes

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42
Q

What is the management for Pernicious anaemia?

A

Medical: Cyanocobalamin PO

or

Medical: Hydroxycobalamin IM

Inject 3 times weekly for 2 weeks then every 3 months

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43
Q

Why should you treat B12 deficiency before folate deficiency?

A

Treating patients with folic acid when they have a B12 deficiency can lead to subacute combined degeneration of the cord

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44
Q

What are the causes of microcytic anaemia?

A 
Iron-deficiency 
Anaemia of chronic disease (normally normocytic)
Alpha thalassaemia 
Beta thalassaemia 
Hereditary spherocytosis
Sideroblastic anaemia 
Lead poisoning
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45
Q

What is the total Iron content of the body?

A

3-4g

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46
Q

What are the causes of iron-deficiency anaemia?

A

Increased requirements: Pregnancy

Increased iron loss: GI bleeding; Menorrhagia

Reduced uptake: Malabsorption; Poor diet

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47
Q

What investigations would you conduct in a patient with suspected anaemia?

A 
FBC 
TFTs 
LFTs
Blood film
Iron 
Ferritin 
Transferrin levels
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48
Q

How do you manage iron-deficiency anaemia?

A

Supportive: Identify and Tx cause
+
Medical: Ferrous fumarate PO

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49
Q

What is the pathophysiology of anaemia of chronic disease?

A

Chronic disease has chronic inflammation (increased by IL-6) thus increased hepcidin resulting in reduced iron absorption

Reduced RBC survival
Reduced EPO
Reduced erythropoiesis

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50
Q

How is anaemia of chronic disease treated?

A

Identify cause and Tx cause

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51
Q

Which populations is ß-thalassaemia most prevalent in?

A

Mediterranean
African
SE Asian

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52
Q

What are the clinical features of ß-thalassaemia?

A

Present within first year of life when HbF changes to normal Hb (includes Beta chains)

Extramedullary haematopoeisis:
Hepatomegaly
Splenomegaly
Skeletal abnormalities

Iron overload:
Growth failure
DM
Hypothyroidism 
Hypogonadism
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53
Q

What is the gold standard investigation for ß-thalassaemia?

A

Hb electrophoresis

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54
Q

How is ß-thalassaemia managed?

A

Blood transfusions

±
Surgery:
- Splenectomy
- HSCS transplant

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55
Q

What is the inheritance of Sideroblastic anaemia?

A

X-linked or acquired

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56
Q

Beta globin gene is present on which chromosome?

A

11

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57
Q

Alpha globin gene is present on which chromosome?

A

Chromosome 16

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58
Q

What are the four potential types of alpha thalassaemia? Give the genotypes.

A

Chromosome 16 contains two genes for alpha Hb.

aa/a- = alpha thalassaemia minima

a-/a- or aa/– = alpha thalassaemia trait

a-/– = HbH disease with tetramers of beta globin chains causing moderate to severe haemolytic anaemia

–/– = Hb Barts syndrome, foetal death

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59
Q

What is the most severe form of alpha thalassaemia?

How many copies of HbAlpha gene are inherited?
How many are functional?

What occurs?

A

Hb Barts Syndrome

Inherit 4 copies, 0 are functional

Foetal death as four gamma goblin chains which cannot carry oxygen due to having a higher oxygen affinity than tissues thus does not deliver oxygen.

Causes extramedullary erythropoiesis leading to severe anaemia, cardiac failure, oedema –> Hydrops foetalis

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60
Q

What are the clinical features of HbH syndrome?

A

Highly variable

Pallor
Anaemia
Jaundice (unconjugated due to chronic haemolytic anaemia)
Gallstone disease (chronic haemolysis)
Extramedullary haematopoiesis: hepatosplenomegaly, skeletal changes
Osteopenia/osteoporosis
Aplastic/hypoplastic crisis: sudden reduction in red blood cell counts
Leg ulcers

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61
Q

What is the diagnostic testing for alpha thalassaemia?

A

Hb analysis by HPLC or electrophoresis

Genetic testing

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62
Q

How is HbH disease managed?

A

Medical: Folic acid; Transfusions ( if <70g/L); Desferrioxamine (if iron overloaded)
±
Surgery: Splenectomy

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63
Q

The beta globin gene cluster is present on which chromosome?

A

11

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64
Q

What are the types of beta thalassaemia?

A

Beta thalassaemia trait (b+/b OR b0/b) = one abnormal gene, either reduced production or no production - asymptomatic with mild anaemia

Beta thalassaemia intermedia (b+/b+) = two abnormal beta globin genes but both reduced

Beta thalassaemia major (b+/b0 or b0/b0) = two abnormal genes with one being no production - require transfusions

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65
Q

What are the clinical features of beta thalassaemia?

A

Anaemia S + Sx

Jaundice 
Gallstones 
Hypogonadism 
Growth restriction 
Cardiac arrhythmia 
Endocrine disorders
Extramedullary haematopoeisis - facial deformity;

Short limbs
OP
Bony pain

Pulmonary hypertension
Clotting disorders
Leg ulcers

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66
Q

What test is used to diagnose beta thalassaemia?

A

Hb electrophoresis or HLPC

Genetic testing

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67
Q

How is beta thalassaemia managed?

A

Supportive: Blood transfusions (2-3 wees)
+
Medical: Desferrioxamine (if iron overloaded)
±
Surgery: Splenectomy (if symptomatic splenomegaly)

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68
Q

How is sickle cell anaemia inherited?

A

Autosomal recessive

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69
Q

What disease does Sickle Cell disease confer an advantage against and why is this?

A

Protective against malaria as more likely to survive and pass on genes - thus selective advantage to having sickle cell anaemia in areas endemic of malaria

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70
Q

A genotype of HbSS suggests?

A. Sickle cell anaemia

B. Sickle cell disease

C. Sickle cell trait

D. Sickle-thalassaemia

A

A

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71
Q

A genotype of HbSC suggests?

A. Sickle cell anaemia

B. Sickle cell disease

C. Sickle cell trait

D. Sickle-thalassaemia

A

B

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72
Q

A genotype of HbS suggests?

A. Sickle cell anaemia

B. Sickle cell disease

C. Sickle cell trait

D. Sickle-thalassaemia

A

C

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73
Q

A genotype of HbSß0 suggests?

A. Sickle cell anaemia

B. Sickle cell disease

C. Sickle cell trait

D. Sickle-thalassaemia

A

D

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74
Q

What type of mutation causes Sickle haemoglobin?

A. Point mutation causing glutamic acid to be converted to valine

B. Point mutation causing valine to be converted to glutamic acid

C. Deletion mutation

D. Point mutation causing glutamic acid to be converted to lysine

A

A

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75
Q

What is the pathophysiology of Sickle Cell anaemia?

A

Sickle Hb polymerises at low oxygen tension thus forms sickle shaped cells which undergo chronic haemolysis and vaso-occlusion of BVs

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76
Q

What can increase the risk of a Sickle Cell crisis?

A

Anything which causes low oxygen levels, replicating low oxygen tension

Dehydration
Infection
Cold exposure
Acidosis

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77
Q

What may vaso-occlusion as a result of sickle cell crisis cause?

A 
Acute painful episodes (i.e. painful crisis)
Acute chest syndrome (i.e. chest crisis)
Renal infarction
Bone infarction or dactylitis (inflammation of a digit)
Myocardial infarction
Stroke
Venous thromboembolism
Priapism (persistent, painful erection)
78
Q

Give 3 examples which may cause an acute drop in Hb in Sickle Cell Disease.

A

Splenic sequestration

Hyperhaemolysis

Aplastic crisis

79
Q

Vaso-occlusive phenomena can lead to which two broad umbrella conditions?

A

Painful episodes

Acute chest syndrome

80
Q

How is Sickle Cell Disease diagnosed?

A

Hb electrophoresis

81
Q

How do you manage sickle cell anaemia?

A

Supportive: Fluids; Analgesia; Oxygen; Blood transfusions; Pneumococcal vaccine (every 5 years)
+
Medical: Hydroxyurea (increases HbF levels)
–> >3 painful episodes; Acute chest syndrome requiring transfusion

± Infection
Medical: ABX

± Neuro complications
Intervention: Exchange transfusion

82
Q

What is the indication for Hydroxyurea in Sickle Cell Disease?

A

≥3 painful episodes requiring hospital

or

Acute chest syndrome requiring transfusion

83
Q

How is G6PD deficiency inherited?

A

X-linked

84
Q

What genetic process means females may display G6PD deficiency?

A

Lyonisation

85
Q

State 3 precipitants of G6PD deficiency.

A

Medications: ABX; Antimalarials; Dapsone; Methylene blue; Sulfonylureas; Rasburicase

Fava beans

Infection

86
Q

What investigation allows diagnosis of G6PD deficiency?

A

Genotype

Assessment of G6PD enzyme activity

87
Q

What is the management of G6PD deficiency?

A

Avoid precipitants of oxidative injury

88
Q

What type of surgery may precipitate B12 deficiency?

A

Gastrectomy - parietal cells produce IF

Ileal resection - absorbed in terminal ileum

89
Q

What are the clinical features of B12 anaemia?

A

Normal anaemia S + Sx

Neuropsychiatric complications…
Peripheral neuropathy
Subacute degeneration of the cord
Focal demyelination

Depression
Personality change
Memory loss

90
Q

What is the management of B12 deficiency?

A

Cobalamin PO

or

Hydroxycobalamin IM

91
Q

What are the clinical features of subacute degeneration of the cord?

A

B12 deficiency with lateral and posterior columns of spinal cord degenerated

AND

Numbness
Weakness
Paraesthesia: loss of vibrational sense; loss of proprioception and ataxia

Ataxia
Sensory deficit
Autonomic dysfunction

Loss of vibration and proprioception
Hyperreflexia
Positive Babinski Sign

92
Q

What is the management of Folate deficiency?

A

Folic acid PO 5mg for 4/12

93
Q

Give a medication which may cause non-megaloblastic anaemia.

A

Azathioprine

Cyclophosphamide

Hydroxyurea

94
Q

How is VWD inherited?

A

Autosomal dominant

95
Q

How is diagnosis of VWD achieved?

A

Clinical diagnosis

96
Q

How do you manage VWD?

A

Desmopressin
/
VWF + Factor VIII (infuse with VWF)

Women (menorrhagia)

  • TXA
  • Mefanemic acid
  • COCP
  • Coil
97
Q

What is the cause of Haemophilia A?

A

Mutation of Factor 8

98
Q

What is the cause of Haemophilia B?

A

Mutation of factor 9

99
Q

How are the Haemophilias inherited?

A

X-linked recessive

100
Q

What are the clinical features of Haemophilia?

A

Spontaneous haemorrhages

Haematomas

Recurrent bleeding
Haemarthrosis

101
Q

How is Haemophilia diagnosed?

A

Bleeding scores
Coag factor assays

Genetic testing

102
Q

How is Haemophilia managed?

A

IV infusion factor 8/9

Acute:
Desmopressin + Infusion of factors + TXA

103
Q

What age does ALL tend to occur?

A

0-4 years old

104
Q

Which of the following is the most common gene mutation seen in ALL affecting children?

A. t(9;22)

B. t(12;21)

C. t(4;11)

D. Hyperdiploid karyotype

A

B

105
Q

What are the clinical features of ALL?

A

Anaemia

Neutropenia

Thrombocytopenia

Tissue infiltration - lymphadenopathy; hepatosplenomegaly; bone pain; mediastinal mass; testicular enlargement

Leucostasis (∆ mental state; headache; SOB; visual changes)

106
Q

What is the diagnostic test for ALL?

A

BM aspirate and biopsy

107
Q

A blood film showing blast cells is suggestive of what cancer?

A

ALL

108
Q

How is ALL managed?

A

Specialist referral + MDT decision
+
Chemotherapy (induction and maintenance therapy)

109
Q

What are the complications of ALL?

A

Tumour Lysis Syndrome

Neutropenic Sepsis

SVCO

Chemotherapy side effects

110
Q

What is the precursor to CLL?

A

Monoclonal B cell lymphocytosis

111
Q

What are the clinical features of CLL?

A 
Weight loss
Night sweats
Fevers
Lethargy 
Anorexia

Lymphadenopathy
Hepatomegaly
Splenomegaly

112
Q

What is the main difference between CLL and MBL?

A

Both have raised B lymphocyte count however MBL is asymptomatic

113
Q

How is CLL diagnosed?

A

Flow cytometry

114
Q

Smudge cells are a feature of which haematological condition?

A

CLL due to so much production but weaker due to vimentin quality/quantity

115
Q

How may you stage CLL?

A

Binet Staging - number of lymphoid sites affected

Stage A = <3 sites

Stage B = >3 sites

Stage C = presence of anaemia and/or thrombocytopenia

116
Q

How is CLL managed?

A

Supportive: Watch and wait (3 month assessment); vaccination; ABX for infection; IvIG (if hypogammaglobulinaemia)

± BM failure; Splenomegaly; Symptomatic; Autoimmune complications
- Chemotherapy

2nd line
Surgery: AlloSCT

117
Q

The formation of an aggressive lymphoma from a CLL is called?

A

Richter Transformation

118
Q

What are the RFs for AML?

A 
MDS
Congenital disorders e.g. Downs Syndrome
Radiation exposure 
Previous chemotherapy
Toxins (organochlorine insecticides; benzene)
119
Q

What age is AML most prevalent in?

A

70s

120
Q

The presence of Auer rods are suggestive of what condition?

A

AML

Myelodysplastic syndrome

121
Q

What is the gold-standard for diagnosis of AML?

A

BM aspirate

122
Q

What is the management of AML?

A

Supportive: referral; education; support
+
Chemotherapy

±
Surgery: AlloHSC transplant

123
Q

What cytogenic finding is most related to CML?

A

t(9;22) - Philadelphia chromosome

124
Q

What are the phases of CML?

A

1) Chronic phase: non-specific symptoms of fatigue, weight loss and night sweats
2) Accelerated phase: blasts in the bone marrow; basophils in blood; persistent thrombocytopenia
3) Blast crisis: Resembles an acute leukaemia with rapid expansion of blasts (>30%) and extramedullary blast proliferation

125
Q

How is CML managed?

A 
Supportive: patient education; support
\+
TKIs: Imatinib/Dasatinib
\+
Chemotherapy

±
Surgery: AlloSCT (in chronic phase if other Tx fail)

126
Q

Give 5 causes of Polycythaemia Vera

A

Primary polycythaemia

Secondary polycythaemia

  • Smoking
  • CLD
  • Obesity
  • OSA
RCC
Wilms' Tumour
Adrenal tumours 
Illicit EPO use
Androgen use
Uterine fibroids
127
Q

What gene mutation causes Polycythaemia Vera?

A

JAK2 gene

128
Q

What are the clinical features of Polycythaemia Vera?

A 
Headache
Visual disturbance
Tinnitus
Itching (especially in a warm bath)
Fatigue
Vertigo
Paresthesia
Plethora
Bruising
Excoriation
Conjunctival injection
Splenomegaly
Erythromelalgia
129
Q

How may Masked Polycythaemia Vera occur?

A

Iron-deficiency anaemia with the background of Primary Polycythaemia Vera

130
Q

How is Polycythaemia Vera managed?

A

Venesection: 200-500mL per time
–> aim for Hct <0.45
+
Low-dose aspirin: 75mg PO OD

± High-Risk (>65 years or thrombotic events)
Medical: Hydroxycarbamide

131
Q

A triad of hepatomegaly + abdominal pain + ascites is part of?

A

Budd-Chiari Syndrome

132
Q

How does an IVC filter work?

A

Inferior vena cava filters are devices inserted into the inferior vena cava, designed to filter the blood and catch any blood clots travelling from the venous system, towards the heart and lungs. They act as a sieve, allowing blood to flow through whilst stopping larger blood clots. They are used in unusual cases of patients with recurrent PEs or those that are unsuitable for anticoagulation.

133
Q

Which cells are seen in Hodgkin Lymphoma?

A

Reed-Sternberg cells - multinucleate cells

134
Q

What is associated with Hodgkin Lymphoma?

A

EBV
Smoking
HIV
Immunosuppression

135
Q

What are the clinical features of Hodgkin’s Lymphoma?

A

lymphadenopathy (75%) - painless, non-tender, asymmetrical

systemic (25%): weight loss, pruritus, night sweats, fever (Pel-Ebstein)

alcohol pain in HL

normocytic anaemia, eosinophilia

LDH raised

136
Q

What are the types of Hodgkin’s Lymphoma?

A

Nodular sclerosing: F; lacunar cells; 70%

Mixed cellularity: large number of Reed-Sternberg cells

Lymphocyte predominant = best diagnosis

Lymphocyte depleted = worst prognosis

137
Q

How is Hodgkin’s Lymphoma staged?

A

Ann-Arbor staging

1 = single lymph node

2 = >2 LN on same side of diaphragm

3 = nodes both sides of diaphragm

4 = spread beyond lymph nodes

A = pruritus

B = other B symptoms

138
Q

How is Hodgkin’s Lymphoma diagnosed?

A

Lymph node biopsy (FNA/Core Biopsy)

139
Q

How is Hodgkin’s Lymphoma managed?

A

Mnemonic: ABVD

Chemotherapy: Doxorubicin + Bleomycin + Vinblastine + Dacarbazine

140
Q

What is the MOA of Doxorubicin?

A

Anthracycline inhibiting topoisomerase II resulting in DNA inhibition and RNA synthesis

141
Q

What are the side effects of Doxorubicin?

A

Cardiomyopathy
Myelosuppression
Skin reactions

142
Q

What is the MOA of Bleomycin?

A

Inhibits DNA synthesis

143
Q

What are the side effects of Bleomycin?

A

Pulmonary fibrosis

Severe idiosyncratic reaction (hypotension, confusion, fever, wheeze)

144
Q

What is the MOA of Vinblastine?

A

Inhibit microtubule formation by binding tubulin

145
Q

What are the side effects of vinblastine?

A

Peripheral neuropathy

Bladder atony

146
Q

What is the MOA of Dacarbazine?

A

Converted to MTIC which acts on guanine, causing methylation

147
Q

What is the side effects of Dacarbazine?

A

Bone marrow suppression

Hepatic necrosis

148
Q

Which of the following is a side effect of doxorubicin?

A. Cardiomyopathy

B. Pulmonary fibrosis

C. Peripheral neuropathy

D. Hepatic necrosis

A

A

149
Q

Which of the following is a side effect of Bleomycin?

A. Cardiomyopathy

B. Pulmonary fibrosis

C. Peripheral neuropathy

D. Hepatic necrosis

A

B

150
Q

Which of the following is a side effect of Dacarbazine?

A. Cardiomyopathy

B. Pulmonary fibrosis

C. Peripheral neuropathy

D. Hepatic necrosis

A

D

151
Q

Which of the following is a side effect of Vinblastine?

A. Cardiomyopathy

B. Pulmonary fibrosis

C. Peripheral neuropathy

D. Hepatic necrosis

A

C

152
Q

Why should patients with Hodgkin’s Lymphoma receive irradiated blood?

A

reduce the risk of transfusion-associated graft-versus-host disease

153
Q

What are the RFs for Non-Hodgkin’s Lymphoma?

A

Elderly
Caucasians
History of viral infection (specifically Epstein-Barr virus)
Family history
Certain chemical agents (pesticides, solvents)
History of chemotherapy or radiotherapy
Immunodeficiency (transplant, HIV, diabetes mellitus)
Autoimmune disease (SLE, Sjogren’s, coeliac disease)

154
Q

How is Non-Hodgkin’s lymphoma staged?

A

Ann-Arbor staging

1 = single lymph node

2 = >2 LN on same side of diaphragm

3 = nodes both sides of diaphragm

4 = spread beyond lymph nodes

A = pruritus

B = other B symptoms

155
Q

How is Non-Hodgkin’s Lymphoma managed?

A

Mnemonic: R-CHOP

Rituximab (mAb to CD20) 
\+
Cyclophosphamide 
\+
Doxorubicin 
\+
Vincristine 
\+
Prednisolone
156
Q

What is the MOA of Rituximab?

A

mAb against CD20

157
Q

What are the side effects of Rituximab?

A

Infusion reaction
Hepatitis B reactivation
Progressive Multifocal Leucoencephalopathy

158
Q

What is the MOA of Cyclophosphamide?

A

Inhibits cross-linking of DNA thus stops DNA synthesis

159
Q

What are the side effects of Cyclophosphamide?

A

Nephrotoxic/Bladder toxic

160
Q

What are the side effects of Methotrexate?

A

Nephrotoxic
Myelosuppression
Idiopathic Pulmonary Fibrosis

161
Q

What is the side effects of Asparagine?

A

Neurotoxicity

Pancreatitis

162
Q

What are the side effects of Cisplatin?

A

Ototoxic

Nephrotoxic

163
Q

What are the side effects of Vincristine?

A

Peripheral neuropathy

164
Q

What are the side effects of Bleomycin?

A

Pulmonary fibrosis

165
Q

What are the complications of Non-Hodgkin’s Lymphoma?

A 
Bone marrow infiltration 
SVCO 
Spinal Cord Compression
Metastasis 
Complications from treatment
166
Q

In Primary myelofibrosis, what is the proliferating cell line?

A

HSCs

167
Q

In Polycythaemia Vera, what is the proliferating cell line?

A

Erythrocytes

168
Q

In essential Thrombocythaemia what is the proliferating cell line?

A

Megakaryocytes

169
Q

Which form of cancer may Myeloproliferative Disorders become?

A

AML

170
Q

What is myelofibrosis?

A

Myelofibrosis is where the proliferation of the cell line leads to fibrosis of the bone marrow. The bone marrow is replaced by scar tissue. This is in response to cytokines that are released from the proliferating cells. One particular cytokine is fibroblast growth factor. This fibrosis affects the production of blood cells and can lead to anaemia and low white blood cells (leukopenia).

The scar tissue inhibits haematopoeisis in the marrow thus extramedullary haematopoiesis leads to HSM which can cause portal hypertension or SCC

171
Q

Tear-drop shaped RBCs are seen in which blood disorder?

A

Myeloproliferative disorders

172
Q

What is the gold-standard diagnosis for myeloproliferative disorders?

A

Bone marrow biopsy

173
Q

How is Primary Myelofibrosis managed?

A

Supportive: treat anaemia; splenomegaly; portal hypertension
±
Surgery: AlloHST tranplant

±
Chemotherapy

174
Q

How is Polycytheamia Vera managed?

A

Supportive: Aspirin + Venesection
±
Chemotherapy

175
Q

How is Essential Thrombocythaemia managed?

A

Medical: Aspirin
+
Chemotherapy

176
Q

What is myelodysplastic syndrome?

A

myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells. There are a number of specific types of myelodysplastic syndrome.

177
Q

What are the clinical features of myelodysplastic syndrome?

A

Anaemia
Neutropenia
Thrombocytopenia

178
Q

How is Myelodysplastic Syndrome managed?

A

Bone marrow aspiration and biopsy

179
Q

How is Myelodysplastic Syndrome managed?

A 
Supportive: Watchful waiting; Transfusion 
±
Chemotherapy 
±
Surgery: HSC transplant
180
Q

What is multiple myeloma?

A

haematological malignancy characterised by plasma cell proliferation

181
Q

What are the clinical features of Multiple Myeloma?

A

Mnemonic: CRABBI

Calcium (hypercalcaemia): bones, groans, psychiatric moans, stones

Renal (monoclonal production of Ig): dehydration, thirst, amyloidosis, nephrocalcinosis, nephrolithiasis

Anaemia (BM crowding): fatigue and pallor

Bleeding (thrombocytopenia): bruising and bleeding

Bones (BM infiltration): fragility fractures

Infection (reduced normal number of immunoglobulins): Recurrent infections

182
Q

Bence Jones Protein is present in which haematological condition?

A

Present in Multiple Myeloma

183
Q

What is the management of Multiple Myeloma?

A

Induction therapy: Bortezomib + Dexamethasone
+
Surgery: HSC Transplant

184
Q

What are the complications of multiple myeloma?

A 
Pain 
Pathological fractures
Infection
VTE prophylaxis
Fatigue
185
Q

What conditions are associated with pernicious anaemia?

A

Hypothyroidism
Vitiligo
Addisons
Atrophic gastritis

186
Q

What are the indications for Hydroxyurea in the management of a vaso-occlusive crises in Sickle Cell Anaemia?

A

3 or more admissions
Cx syndrome
Previous stroke

187
Q

What are the components of a haemolytic screen?

A

Reticulocytes
LDH
Haptoglobulin
Direct anti-globulin test

188
Q

What are the clinical features of a thrombotic crisis in Sickle Cell Disease?

A

‘Painful crisis’ as it is painful thus thrombosis occludes the vessel

Can be caused by: infection, dehydration, deoxygenation

Clinical DDx

189
Q

What are the clinical features of a sequestration crisis?

A

Sickling within the organs worsening the anaemia

Increased reticulocyte count

190
Q

What are the clinical features of acute chest syndrome in Sickle Cell Disease?

A

Vaso-occlusion in the pulmonary microvasculature resulting in infarction of the lung parenchyma

Cx pain
SOB

CXR: pulmonary infiltrates

191
Q

What is an aplastic crises in Sickle Cell Disease?

A

Parvovirus infection resulting in aplastic anaemia - Hb drop ± thrombocytopenia ± neutropenia

Reduced reticulocyte count

192
Q

What is the clinical features of a haemolytic crises in Sickle Cell Anaemia?

A

Features of jaundice

Increased rate of haemolysis
Schistocytes seen on blood film

Medicine (18 decks)

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